Principles of treatment
• Chemotherapy
oTyrosine kinase inhibitors
oOther myelosupressive drugs (alfa-interferon, hydroxyurea, etc)
•Stem cell transplantation
Mechanism of action of Imatinib
2012 |
2013 |
2014 |
Male, 65 years, engineer |
|
hospitalization |
General weakness
Fever
Weight loss
Heaviness in right hypochondrium
Hepatomegaly
Peripheral lymphadenopathy
CBC
WBC Promyelocytes Myelocytes Metamyelocytes Hb
38 |
37,2 |
38,1 |
37,3 |
38,2 |
37,1 |
|
|
|
-10 |
-6 kg |
|
kg |
||
|
30х109/l |
Ph-chromosome+ |
50х109/l |
|
|
2% |
|
23% |
|
20% |
110 g/l |
100 g/l |
Chronic lymphocytic leukemia
•В-cell prolipherative disease
•Proliferation of mature but functionally incompetent lymphocytes expressing CD19, CD23, CD20,CD5-markers
•Association with immune deficiency and viruses.
Epidemiology and etiology of CLL
•The most common leukemia in adults in Western countries: 30% of all leukemias
•Median age 69 years
•M/F 1,5 : 1,0
•Family history is important
•Often asymptomatic
•Association with immunodeficiency and viral infections
Clinical presentation
25-50 % asymptomatic at early stages
1.Lymphoproliferative syndrome
•Progressive lymphadenopathy
•Splenomegaly, hepatomegaly
2.Constitutional symptoms
•Fever, malaise, swellings, weight loss
3.Signs of tumor proliferation
4.Specific features (repeated infections, autoimmune hemolytic anemia, thrombocytopenia)
Classification of CLL (Binet) (1989)
Based on the number of involved regions
Diagnostic criteria
1.Absolute lymphocytosis > 5х109/л with < 55% of prolymphocytes
2.Presence of CD19, CD20, CD23,and CD5 on B-cells (peripheral blood flow cytometry)
3.Lymphocytes should dominate in bone marrow (>30% of nuclear cells)